Aortic Stenosis / Hypoplastic Left Heart Syndrome at 20 weeks - any experiences?

[LittleMy34]

Hello all,

Have just had a foetal heart scan at 20 weeks following a high nuchal translucency measurement, and unfortunately they found that the baby has severe aortic stenosis - a narrowing of at least one and possibly two of the main arteries. They seem to think that it's fairly likely to lead to Hypoplastic Left Heart Syndrome by the time the baby is born, which is very severe and the baby would die within a few days without major surgery.
If we go ahead, the baby would need surgery within a day or two of birth, followed by two more ops before he reaches school age. the fist op carries a 30% mortality risk, the second two are less severe.

So now we're trying to decide what to do - which is proving a bit difficult, to say the least..... Neither option seems like the obvious thing to do.

anyone had this experience? how on earth did you go about making a decision?

any thoughts welcomed.

LittleMy

A good friend of mine has a dd who was found to have her aorta attached to the right ventricle at 5wks old. Not the same condition, however, life threatening. They went ahead with the aortic switch operation and their dd was in ICU for a couple of months, she is now 3yrs and is doing extremely well, happy and healthy.

It is a very difficult decision, but as they say, 'It is better to have loved and lost than to never have loved at all' x
Meaning that if you don't go ahead you will not get the opportunity to know what may be.
Very for your news.

My DH was born with this condition in 1968. He was a 28 week twin who weighed just 2lbs. Sadly his twin did not survive. He had several operations in childhood and has to have an MRI every year as he may require a valve replacement operation at some point in the future. But he is happy, healthy and looking forward to his 40th birthday this year.

Ds' best pal had aortic stenosis, although I think it cannot have been as severe as the scenario you've been given.
He did very well in surgery as a newborn and apart from the occasional follow-up with a cardiologist he is as fit and well as any other child we know.
What a terrible blow for you all, LittleMy. Can the scanning hospital arrange for you to meet a paediatric surgeon in the field to explain the operation and prognosis in more detail to you, or have you already had such a meeting? Do you have a clear idea of where your baby would be admitted for this surgery?
Antenatal Results and Choices (ARC) provide counselling and advice for people considering termination following in utero diagnosis. Perhaps they can give you some help.
HLHS used to be invariably fatal. So much has been done to make it possible for babies with this condition to have a reasonable chance of a good life after surgery.
Thinking of you and good luck with your decision.

There is a charity Little Hearts Matter based in Brum which has vast experience of Hypoplast. Contact them as they have great knowledge and will talk you thro' every thing. Best Wishes

Thanks for all your messages - we have stacks of information about it all - the problem is in knowing how on earth to make the decision. Either we terminate, which is a certain outcome, or we put our son through three traumatic and invasive surgical procedures, one within days of birth, with overall only a 70% chance of survival. plus he would then have to be careful of endocarditis for the rest of his life, and probably face more operations as he got older to replace shunts etc as he outgrew them.

and on top of all that, there's the possibility that his heart might fail anyway and he would need a transplant, which he may not be able to get.

but he could lead a relatively normal life and go on to have family of his own.

how on earth does one choose between those two options!
sorry, am feeling a bit lost.

Do you have other children to consider LittleMy?

Yes - DS1 is nearly two. That's the other complication - how will he cope if we're spending weeks at a time up at Guy's in London (we're in Bristol) and he has to be at Grannie's or at home with one parent while the other one is up in London?

Plus the potential trauma of losing a sibling later on in life, and the fact that certainly until this baby is around three, this would rule our lives completely.

But then I wonder if I'm using that as an excuse to cop out of it all.

yikes.

I don't see it as an excuse, I see it as a valid factor. Your ds1 is too young to understand why all the disruption is happening. A child of four and over would be capable of better acceptance of the situation.
On the other hand it is possible that ds1 would in time forget it all.
What you are experiencing here is one of the downsides of excellent antenatal screening procedures, I am so sorry you are faced with this dilemma.
I wish someone with better understanding of your situation was around to talk to you. Keep bumping because we have people on here whose children have had huge surgery as newborns, and people whose decision was to terminate the pregnancy. Mn is the sort of environment where support is there whatever path you take. The charity OhNo mentions sounds especially worth contacting.

Thanks marina, it makes me feel better to know that you don't think I'm just copping out. Not that terminating would be the easy option, but I don't want to always wonder what the outcome would have been for our little boy if we'd gone ahead.

It's all too difficult - but DS1 will be home from nursery in a minute, so am going to try and forget about it for a while and concentrate on him!

Thank you for your kind words - much appreciated.

Just bumping this for you Littlemy

I don't know what to say to you as I have not come across this before but surely a 70% chance of survival has got to be worth giving him a chance? Are you sure that if you terminated now you wouldn't be thinking "what if".

I don't envy you this decision at all.

What an awful situation for you, LittleMy.

I was told my DD had a brain abnormality when I was about 24 weeks pregnant, and we were referred to a neurosurgeon to discuss shunt surgery. As things turned out, DD was fine but it was extremely helpful to meet the surgeon. The comment that stuck with me in particular was that they treat the child, not the scan.

I spoke to a counsellor at LIFE, which is an RC charity, as I wanted to talk to someone who understood how frightened I was, without being steered down the amnio/late termination path, which I knew in my heart wasn't the answer for me but I was scared that in my panic I would bolt down it. They were extremely helpful and completely understood how frightened I was for my baby, myself and the rest of my family.

Rolf/MrsDarcy

I have a friend whose second child had HLHS which wasn't diagnosed antenatally. He went home from hospital fine, but after about a week he was rushed back in and resuscitated. His surgeon said his aorta was one of the smallest he'd ever operated on when he did the first stage of surgery. Sadly, he didn't survive but many children do nowadays. Wishing you strength at this difficult time, xxx

littlemy I work in a children's cardiac unit so have experience of caring for infants and children with hypoplastic left heart
All I can say to you is that you need to make the decison that is right for you and your family and not for anyone else
At the unit where I work the last research we did showed that about 40% of parents chose to terminate and 60% to carry on with the pregnancy
I am not going to say anymore but I would advise you to contact the children's heart federation helpline they can put you in touch with other parents who have gone through similar situations. I don't think anyone can advise you unless they have been through it themselves as it is a very difficult decision to make
their contact details are here
they have a helpline that you can call
take care this is a really hard time for you and your dh please get as much help and support as you need

Thanks for all your thoughts and help.

finallypregnant - if it was just the 70% survival rate, I think we'd go for it no problems. The trouble is, that's just the rate of getting through the operations - there's still the possibility of heart failure, plus reduced life expectancy etc. Plus I don't know how I feel about putting such a tiny infant through incredible major surgery - I've always been of the opinion that there's a point at which further medical treatment becomes too much to bear - I'm just not sure what that point is!

Rolf - that's interesting to hear, I do feel the doctors are steering us down the termination route, whether consciously or unconsciously. Will seek out some people to talk to.

andie - thank you for your wise words - would you be able to tell me, how do the children in the cardiac unit cope? I'm very worried about putting a child through such traumatic surgery, i saw my dad go through valve replacement surgery and it was pretty awful to be honest.

feeling much calmer, but still have no idea what to do!

littlemy, I remember talking to my friend about the surgery and she too felt that had her son survived his life would have been filled with hospital visits and a limited life expectancy. She also worried about the impact on her older daughter. Please take your time and be kind to yourself, xx

thanks bundle, that's exactly what I'm afraid of. We're talking about a child which without drastic medical intervention, would certainly die - I just wonder at what point we should decide to let nature do what it does best, and not to intervene.

but I feel like I'm letting my child down if I don't fight for it to live.

My grandmother had a baby who died at two days old, probably from the same problem, but this was back in the early 1930s so completely undiagnosed. In some ways I wonder if it was easier then, when there weren't all these options available and choices were much more limited!

exactly littlemy, the technology does make it harder in lots of ways. but having said that my friend and her family were absolutely certain they were right to give him the opportunity via the surgery to have a chance at life and I'm sure they don't regret it. xxx

Oh good, more helpful and detailed posts (was hoping you'd see this andie and bundle)
It's a different medical condition but some friends ds1 was born with artery and liver problems (undetected in pregnancy) and had two major operations as a young baby. Sadly, like bundle's friends' baby, he did not make it, but I know they do not regret giving him a chance of a life beyond infancy

Same thing happened here - we were given a diagnosis of hypoplastic left heart at our 20 week scan.

Between our 20 week scan and our referral to Kings for fetal cardiac scans - We talked about it and decided that no matter what this was our baby and she would be born - we would deal with the rest one day at a time. That is the decision that we made. We know that others in our position have not made that decision.

I know when we were looking into it there was a brilliant group in the US that we were able to get a lot of information from.

Our outcome - after being sent to Kings for further fetal cardiac scans - we learnt our daughter did not have HPLH but a complete AVSD ( hole between aortas and hole between ventricles) and down syndrome.

She had two open heart surgeries at 8 weeks old. She is at risk of endocarditis and will be for life. We just make sure it is known that she needs antibiotics prior to surgery. She has annual visits for the cadiologist but we do have a lot of other visits to hospital for other reasons.

TOday I say goodbye as that little babe goes off to school for the first time!! (pic on my profile)

IT was tough watching her go through such invasive surgery and to think how on earth those surgeons are able to operate on such tiny tiny hearts. But she was ( and still is) a fighter and she is doing so well now.

LittleMy34 - hello - congratulations on your pregnancy. My dd has a rare congenital heart defect (not HLHS). We didn't know about it before she was born so were never faced with the decision whether or not to continue with the pregnancy. I also have older children, ds1 was 5 and ds2, 2 when we had dd.

dd had major heart surgery as a baby and spent a long time in hospital and ICU away from our home. She would have been considered for transplant but at the time was ironically too poorly. We have had much uncertainty over her future; she has only had the one surgery, isn't 'fixed' but is finely balanced at the moment and managing well on medication. She has had several other cardiac procedures as a toddler and young child and has coped well.

I can't tell you what to do, what is the right decision. I do have personal knowledge of many children with HLHS and some with even more complex conditions, they don't all make it that's true, but many of them are living full lives despite being 'life limited'.

Someone has mentioned Little Hearts Matter, also the Children's Heart Federation and Heartline. There is a very active message board at Heartline where you will find a pregnancy board also the message board at Little Hearts Matter has an antenatal diagnosis section.

I have to say that some doctors are not very up to date with the advances in surgery for HLHS - 10 or 12 years ago it was considered unsurvivable but there have been great forward steps with the treatment. However, it is still a very difficult path to follow. Try to speak with a paeds cardiac surgeon if you can. You may be able to chat with a Cardiac Liasion nurse if you call Guys.

I know I won't have helped you much here particularly - it is such a personal decision - if you have anymore questions I'll try to answer them if I can.

Heartline message board.The link to the Heartline main page is at the bottom.

Little Hearts Matter

Children's Heart Federation

very helpful

we found talking with other parents who had been there done that so to speak very very helpful.

parent site

another parent groupthis is the one mentioned by ohnoherewego.

As dd1 was our first we did not have to consider other siblings BUT we did not intend for her to be an only child. Dd2 who is 3 - things going to hospital for appts to see various consultants is what you do. She knows that there are some children who are born with things that doctors need to check up or things that need to be fixed ( she is 3 so simple explantations.)

Oh snap eidsvold!

I was just about to mention Heartline but Saggar has beaten me to it .

My situation was similar to Saggar's in that we didn't know that ds1 had a congenital heart defect until after his birth. He was taken into hospital as an emergency case and of course all I wanted in the world was for him to stay alive. DH and I have often since spoken about what might have happened had we known about his condition before his birth and neither of us would have known what to do in all honesty. Ds1 is now a very happy and healthy 7 year old and to look at him you would never know what he had been through as a baby. We do worry for his future in that it isn't going to be as straightforward as a heart healthy person's would. I remember thinking that at the time of his diagnosis all the tests and precautions sounded so scary but my experience of them since has been quite positive on the whole. Good luck with your decision, it is both a very difficult and personal one.